Cervical nerve root synovial sarcoma in a child with chromosomal (X;18) translocation. Case report and review of the literature.


Autoria(s): de Ribaupierre S.; Vernet O.; Beck-Popovic M.; Meagher-Villemure K.; Rilliet B.
Data(s)

2007

Resumo

We report on an 11-year-old female with a history of cervicobrachialgia and progressive weakness of the right arm. Cervical spine MRI showed an enhancing heterogeneous intradural mass occupying the right C6-C7 foramen. She underwent a right C6-C7 foraminotomy with a complete macroscopic removal of the lesion. Pathological examination revealed a synovial sarcoma. Treatment was completed by chemotherapy and proton radiotherapy, and the girl remained free of symptoms for 3 years. After appearance of new symptoms, a local recurrence was confirmed, and despite aggressive treatment with salvage chemotherapy and radiotherapy, the disease progressed beyond medical control, and the child died, 6 years after diagnosis. Early recognition of this rare entity compared to its more benign differential diagnosis is crucial, as an aggressive management is needed.

Identificador

http://serval.unil.ch/?id=serval:BIB_F52924998479

isbn:1423-0305[electronic]

pmid:17786003

doi:10.1159/000106387

isiid:000249305600007

Idioma(s)

en

Fonte

Pediatric neurosurgery, vol. 43, no. 5, pp. 382-5

Palavras-Chave #Cervical Vertebrae; Child; Chromosomes, Human, Pair 18; Chromosomes, Human, X; Female; Humans; Peripheral Nervous System Neoplasms; Sarcoma, Synovial; Spinal Nerve Roots; Translocation, Genetic
Tipo

info:eu-repo/semantics/article

article